Primary Lateral Sclerosis

Primary Lateral Sclerosis (PLS) is a neurodegenerative disease characterized by a slower progression compared to Amyotrophic Lateral Sclerosis (ALS) and primarily affects upper motor neurons. Its symptoms, which typically begin in the lower extremities, include spasticity, hyperreflexia, bladder instability, and urinary retention. Treatment for PLS, which lacks disease-modifying options, focuses on improving mobility and reducing spasticity through a combination of physical therapy, assistive devices, and medications like baclofen, tizanidine, and clonazepam. Management benefits from a multidisciplinary approach, similar to ALS care, but Riluzole, commonly used in ALS, has not shown significant benefits for PLS.

2024-01-05 14:05:31 - Editor

Overview of Primary Lateral Sclerosis

Primary lateral sclerosis is a progressive isolated upper motor neuron neurodegenerative disease. Compared with ALS, primarily lateral sclerosis is characterised by slower progression and absence of lower motor neuron features. Symptoms usually begin in the lower extremities, with spasticity in gait and hyperreflexia. Many patients also have bladder instability and urinary retention. Additionally, some patients may develop lower motor neuron later in their clinical course of the disease (upper motor neuron-onset ALS). Pure primary lateral sclerosis appears to have a more benign prognosis than ALS.

Multidisciplinary Clinic Approach

Patients with primary lateral sclerosis (PLS) may benefit from evaluation at multidisciplinary clinics, similar to those available for ALS patients. It includes neurologists, physical therapists, occupational therapists, speech therapists, respiratory therapists, dieticians and social workers. There is no disease modifying treatment for primary lateral sclerosis. The goal of treatment is to improve mobility and relieve the discomfort associated with spasticity.

Non-Pharmacological Treatment Approaches

Treatment can be subdivided into non-pharmacological measures and pharmacological treatment. Non-medication approaches include physical and occupational therapy for range of motion exercises, gait, balance training and assistive devices, such as, ankle-foot orthoses, walkers or wheelchairs.

Pharmacological Management

Riluzole has not shown any clear benefit in patients with primary lateral sclerosis. For spasticity, there are several pharmacological approaches. They include baclofen (5 mg 1 to 3 times daily), tizanidine (2 mg daily at bedtime), clonazepam (0.5 mg daily). Also, for pseudobulbar affect, a combination of dextromethorphan/ quinidine (20 mg/ 10mg at a starting dose of one capsule once daily for seven days, then increase to one capsule twice daily) could be beneficial.

References

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